Abstract
The t(8;21) translocation is one of the most frequent chromosomal abnormalities in acute myeloid leukaemia and results in gene fusion between AML1 on chromosome 21 and MTG8 (= ETO or CDR) on chromosome 8. AML1 contains a region of sequence homology to the Drosophila runt gene and the mouse polyomavirus enhancer binding protein PEBP2 alpha gene. The rearrangement occurs within a specific intron of the AML1 gene and results in the formation of a chimaeric protein with the consistent feature that the region of sequence homology of AML1 is fused with almost the entire MTG8 protein. MTG8 (ETO, CDR) is predicted to be a transcription activation factor from its sequence with zinc-finger motifs and proline-rich domains. Thus the rearrangement is a fusion between two probable transcription activation factors.
MeSH terms
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Amino Acid Sequence
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Animals
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Chromosomes, Human, Pair 21 / ultrastructure*
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Chromosomes, Human, Pair 8 / ultrastructure*
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Consensus Sequence
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Core Binding Factor Alpha 2 Subunit
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Core Binding Factor alpha Subunits
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DNA-Binding Proteins / chemistry
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DNA-Binding Proteins / genetics*
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DNA-Binding Proteins / physiology
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Drosophila Proteins
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Drosophila melanogaster / genetics
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Gene Expression Regulation, Leukemic
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Humans
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Leukemia, Myeloid, Acute / genetics*
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Molecular Sequence Data
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Neoplasm Proteins / chemistry
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Neoplasm Proteins / genetics*
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Neoplasm Proteins / physiology
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Nuclear Proteins
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Oncogene Proteins, Fusion / chemistry
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Oncogene Proteins, Fusion / genetics*
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Oncogene Proteins, Fusion / physiology
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Protein Binding
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Protein Structure, Tertiary
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Proto-Oncogene Proteins*
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RUNX1 Translocation Partner 1 Protein
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Sequence Homology, Amino Acid
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Transcription Factor AP-2
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Transcription Factors / chemistry
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Transcription Factors / genetics*
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Transcription Factors / physiology
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Transcription, Genetic
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Translocation, Genetic*
Substances
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Core Binding Factor Alpha 2 Subunit
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Core Binding Factor alpha Subunits
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DNA-Binding Proteins
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Drosophila Proteins
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Neoplasm Proteins
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Nuclear Proteins
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Oncogene Proteins, Fusion
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Proto-Oncogene Proteins
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RUNX1 Translocation Partner 1 Protein
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RUNX1 protein, human
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RUNX1T1 protein, human
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TfAP-2 protein, Drosophila
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Transcription Factor AP-2
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Transcription Factors
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run protein, Drosophila